Predictors of mortality in children with myelomeningocele and symptomatic Chiari type II malformation

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Chiari malformation type II (CM-II) in myelomeningocele is associated with a significant rate of mortality and poor outcome. Death is frequently heralded by the onset or progression of neurological symptoms. The authors sought to identify predictors of poor outcome and mortality within the myelomeningocele population at Children’s Hospital of Pittsburgh.


A retrospective chart and radiology review was performed on all infants who underwent primary closure of a myelomeningocele defect at Children’s Hospital of Pittsburgh between the years of 1995 and 2015. Preoperative symptoms and signs leading to CM-II decompression, as well as operative details and postoperative changes in these symptoms and signs, were investigated in detail and correlated to outcome. Poor outcome was defined as death, stridor, or ventilator dependence. Deceased patients were separately assessed within this subgroup.


Thirty-two (21%) of 153 patients were found to have symptomatic CM-II. Of the 32 patients meeting inclusion criteria, 12 (38%) had poor outcomes. Eight patients (25%) died since initial presentation; 5 of these patients (16% of the overall cohort) died within the 1st year of life and 3 (9%) died during adolescence. Seven (88%) of the 8 patients who died had central apnea on presentation (p = 0.001) and 7 (44%) of the 16 patients who developed symptoms in the first 3 months of life died, compared with 1 (6.3%) of 16 who developed symptoms later in childhood (p = 0.04). The median Apgar score at 1 minute was 4.5 for patients who died and 8 for surviving patients (p = 0.006). The median diameter of the myelomeningocele defect was 5.75 cm for patients who died and 5 for those who survived (p = 0.01). The anatomical level of defect trended toward higher levels in patients who died, with 4 patients in that group having an anatomical level at L-2 or higher compared with 5 of the surviving patients (p = 0.001). The median initial head circumference for the 5 patients dying in the 1st year of life was 41.5 cm, versus 34 cm for all other patients (p = 0.01).


CM-II in spina bifida is associated with a significant mortality rate even when surgical intervention is performed. Death is more frequent in symptomatic patients presenting prior to 1 year of age. Late deaths are associated with symptom progression despite aggressive surgical and medical intervention. In this patient cohort, death was more likely in patients with symptomatic presentation during the first 3 months of life, low Apgar scores, large myelomeningocele defects, early central apnea, and large head circumference at birth.

ABBREVIATIONS CM-II = Chiari malformation type II.

Article Information

Correspondence Michael M. McDowell: University of Pittsburgh Medical School, Pittsburgh, PA.

INCLUDE WHEN CITING Published online March 23, 2018; DOI: 10.3171/2018.1.PEDS17496.

Disclosures The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this paper.

© AANS, except where prohibited by US copyright law.




Akbari SHLimbrick DD JrKim DHNarayan PLeonard JRSmyth MD: Surgical management of symptomatic Chiari II malformation in infants and children. Childs Nerv Syst 29:114311542013


Bell WOCharney EBBruce DASutton LNSchut L: Symptomatic Arnold-Chiari malformation: review of experience with 22 cases. J Neurosurg 66:8128161987


Cesmebasi ALoukas MHogan EKralovic STubbs RSCohen-Gadol AA: The Chiari malformations: a review with emphasis on anatomical traits. Clin Anat 28:1841942015


Chang KHKim DKJun BCPark YS: Temporal bone myeloid sarcoma. Clin Exp Otorhinolaryngol 2:1982022009


Charney EBRorke LBSutton LNSchut L: Management of Chiari II complications in infants with myelomeningocele. J Pediatr 111:3643711987


Coniglio SJAnderson SMFerguson JE II: Functional motor outcome in children with myelomeningocele: correlation with anatomic level on prenatal ultrasound. Dev Med Child Neurol 38:6756801996


el Gammal TMark EKBrooks BS: MR imaging of Chiari II malformation. AJR Am J Roentgenol 150:1631701988


Fletcher JMCopeland KFrederick JABlaser SEKramer LANorthrup H: Spinal lesion level in spina bifida: a source of neural and cognitive heterogeneity. J Neurosurg 102 (3 Suppl):2682792005


Hashiguchi KMorioka TMurakami NTogao OHiwatashi AOchiai M: Sequential morphological change of Chiari malformation type II following surgical repair of myelomeningocele. Childs Nerv Syst 32:106910782016


Kulkarni AVRiva-Cambrin JButler JBrowd SRDrake JMHolubkov R: Outcomes of CSF shunting in children: comparison of Hydrocephalus Clinical Research Network cohort with historical controls: clinical article. J Neurosurg Pediatr 12:3343382013


McLone DG: Continuing concepts in the management of spina bifida. Pediatr Neurosurg 18:2542561992


Mizuguchi KMorota NKubota M: [Respiratory complications in children with Chiari malformation type II associated with myelomeningocele.] No To Hattatsu 48:25282016 (Jpn)


Ogiwara HMorota N: Surgical decompression without dural opening for symptomatic Chiari type II malformation in young infants. Childs Nerv Syst 29:156315672013


Papasozomenos SRoessmann U: Respiratory distress and Arnold-Chiari malformation. Neurology 31:971001981


Park TSHoffman HJHendrick EBHumphreys RP: Experience with surgical decompression of the Arnold-Chiari malformation in young infants with myelomeningocele. Neurosurgery 13:1471521983


Pollack IFKinnunen DAlbright AL: The effect of early craniocervical decompression on functional outcome in neonates and young infants with myelodysplasia and symptomatic Chiari II malformations: results from a prospective series. Neurosurgery 38:7037101996


Pollack IFPang DAlbright ALKrieger D: Outcome following hindbrain decompression of symptomatic Chiari malformations in children previously treated with myelomeningocele closure and shunts. J Neurosurg 77:8818881992


Salomão JFBellas ARLeibinger RDBarbosa APBrandão MA: [Symptomatic Chiari type II malformation.] Arq Neuropsiquiatr 56:981061998 (Portuguese)


Tubbs RSOakes WJ: Treatment and management of the Chiari II malformation: an evidence-based review of the literature. Childs Nerv Syst 20:3753812004


Vandertop WPAsai AHoffman HJDrake JMHumphreys RPRutka JT: Surgical decompression for symptomatic Chiari II malformation in neonates with myelomeningocele. J Neurosurg 77:5415441992


Wickramasinghe S: Posterior fossa decompression in shunt-treated hydrocephalic children. Dev Med Child Neurol 15 Suppl:11131968




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